A variety of conditions overlap with acne and affect the course and effectiveness of treatment. From a treatment standpoint, these conditions most importantly include seborrheic dermatitis and rosacea. From a diagnosis standpoint, important overlaps to recognize include the follicular occlusion tetrad (two or more of the following: cystic acne, hidradenitis suppurativa, dissecting cellulitis, pilonidal cysts), SAPHO syndrome and PAPA syndrome.
This article will review these acne overlaps and suggest therapeutic approaches.
SEBORRHEIC DERMATITIS
Seborrheic dermatitis is a condition that commonly overlaps with acne. Physically this can manifest as a combination of comedones and pustules on the face, chest and back, along with scaling erythematous plaques on the scalp, nasal alae and eyebrows. Hypopigmenation of the eyebrow and nasal alae skin can also be present.
The presence of co-incident sebborheic dermatitis has been suggested as an indicator for poor clinical response in acne patients treated with antibiotics and suggests early use of isotretinoin.1 Specifically, patients with a sebum excretion rate of 2.5 µgrams/cm2/minute experienced, on average, a 17% improvement with their acne in response to 6 months of treatment with topical 5% benzoyl peroxide and an oral agent from among one of the following: oral erythromycin (1 gram/daily), minocycline (100 milligram/daily), oxytetracycline (1 gram/daily) or cotrimoxazole (400 milligram/daily).
Topical Treatments For Seborrheic Dermatitis and Acne
A variety of topical treatments can be used to treat acne and seborrheic dermatitis simultaneously, including ketoconazole and sulfur.
Ketoconazole has antiandrogenic properties and might have utility against acne — which has an androgenic basis — not just seborrheic dermatitis.2
Sulfur has a keratolytic effect due to formation of hydrogen sulfide through a reaction that depends upon direct interaction between sulfur particles and keratinocytes and mild antifungal and antibacterial activity of unknown mechanism.3 Sulfur 5% and sodium sulfacetamide 10% alone or in combination are useful treatments for acne and seborrheic dermatitis.
Azelaic acid is an antikeratinizing agent, displaying antiproliferative cytostatic effects on keratinocytes and modulating the early and terminal phases of epidermal differentiation. It might be useful for sebborheic dermatitis, but there are no reports of azelaic acid use for seborrheic dermatitis.4
Antidandruff shampoos (e.g., tar, zinc, selenium) are helpful agents for this overlap, as they can allow for a treatment of the face and scalp with a wash and concentration of acne treatment of the face with leave-on products.
Oral Treatments For Seborrheic Dermatitis and Acne
Oral treatments can be useful for acne/seborrheic dermatitis overlap.
Isotretinoin is a useful, albeit not curative, treatment for seborrheic dermatitis. Very low-dose isotretinoin is effective in controlling seborrhea.5 Systemic isotretinoin reduces sebocyte lipid synthesis by 75% after 4 weeks, with daily doses as low as 0.1 mg/kg.
Oral ketoconazole has both antiandrogenic properties and is effective against pityrosporum folliculitis.7 This is the case in particular with women with hirsutism, acne and seborrheic dermatitis.8
Hormonal Treatments For Seborrheic Dermatitis and Acne
Hormonal treatments could have a role in treating the overlap of acne and seborrheic dermatitis. Estrogens decrease sebum production by suppressing gonadotrophin release and androgen production and increasing sex hormone-binding globulin production. There are a few studies that suggest that birth control pills with estrogens might be useful treatments for acne and seborrheic dermatitis.9 This is particularly true for the agents chlormadinone acetate, cyproterone acetate and dienogest (not available in the United States), potent, orally active progestogens, which have antiandrogenic, instead of partial androgenic, activity.10
Incompatible Acne/Seborrheic Dermatitis Treatments
Some treatments for acne and seborrheic dermatitis clash and should not be used when these conditions coincide.
Most importantly, topical steroids, which are helpful in the treatment of seborrheic dermatitis — likely by suppressing the inappropriate immune response to Malassezia furfur — but can cause acne. Thus, when these conditions overlap, topical steroids should not be used.
On a different note, antifungal agents that do not have anti androgenic effects — such as Allylamines — should not be used to treat acne/seborrheic dermatitis overlap.
ROSACEA
Classically it is stated that comedones do not manifest with rosacea. Sometimes, however, acne and rosacea do overlap. Rosacea is above all a disease of sensitive skin. This is important to be mindful of when treating acne/rosacea overlap.
Incompatible Acne and Rosacea Treatments
Acne Treatments. Some acne treatments are not appropriate for rosacea patients. Drying or irritating treatments for acne, such as benzoyl peroxide and salicyclic acid, are not well tolerated by patients with rosacea. Similarly, while trichloroacetic acid or beta peels can be used as adjuvants for acne treatment, they are extremely irritating in rosacea patients. Trimethoprim-sulfamethoxazole is a well-reported treatment for acne, but it is not used to treat rosacea, and hence should not be used for acne/rosacea overlap.
Rosacea Treatments. Likewise, there are treatments for rosacea that are not useful for acne and should be avoided in patients with acne/rosacea overlap. These include topical and oral metronidazole, topical tacrolimus or pimecrolimus and topical permethrin.
Appropriate Rosacea/Acne Combination Treatments
Although topical tretinoin is usually poorly tolerated by rosacea patients, adapalene can be tolerated by some rosacea patients and is useful in overlap patients. In fact, a comparison of topical adapalene gel (0.1%) and topical metronidazole gel (0.75%) found a similar effect against the inflammatory lesions of rosacea, but a superior effect in the metronidazole against erythema.11
Many treatments can be used in both acne and rosacea. In particular, oral tetracycline, azithromycin, doxycycline and minocycline are useful treatments for acne/rosacea overlap. Minocycline effectively decreases eyelid bacterial flora in patients with acne rosacea or blepharitis. One of the mechanisms of newer generation tetracycline analogues may be a decrease or elimination of bacterial flora from the eyelids.12 Isotretinoin can be curative for acne, and while only remittive for rosacea, is a very efficacious treatment while used. Similarly, sulfur 5% and sodium sulfacetamide 10%, alone or in combination, are useful treatments for acne and seborrheic dermatitis. Azelaic acid and topical clindamycin are useful for acne/rosacea overlap.
SAPHO
SAPHO syndrome is an acronym for a syndrome consisting in whole or part of synovitis, acne, pustulosis, hyperostosis, and osteitis. SAPHO is thought be an autoimmune disease in the family of psoriasis, rheumatoid arthritis and inflammatory bowel disease.
Joint manifestations of SAPHO mainly involve aseptic osteitis, osteomyelitis or arthritis of anterior chest, sacroiliac joints and long bones. Skin manifestations besides acne include palmoplantar pustulosis, non-palmoplantar pustulosis, psoriasis vulgaris, Sweet syndrome, hidradenitis suppurativa, dissecting cellulitis or pyoderma gangrenosum.
As the acne of SAPHO can be severe, the treatment of choice is isotretinoin; but as the stimulus underlying SAPHO is constant, repeated courses of therapy might be necessary.
PAPA SYNDROME
PAPA syndrome is an acronym for pyogenic arthritis, pyoderma gangrenosum and acne. PAPA syndrome is caused by mutations in proline serine threonine phosphatase-interacting protein [PSTPIP1, or CD2-binding protein 1 (CD2BP1)], a tyrosine-phosphorylated cytoskeletal organization protein interacting with pyrin. In this, it shares a common etiology with Familial Mediterranean Fever.13 Pyrin is a familial Mediterranean fever protein, cytoskeletal associated with myeloid/monocytic cells and modulates IL-1b, NF-kB activation and apoptosis.
An autosomal dominant disease with variable penetrance, PAPA syndrome can manifest in the following ways: (1) early pauciarticular, nonaxial, destructive, steroid-responsive arthritis; (2) pyoderma gangrenosum; (3) severe cystic acne in adolescence and beyond; and (4) acne vulgaris. In PAPA syndrome abscesses at the site of parenteral injections and cytopenia associated with sulfonamide medications, insulin-dependent, adult-onset diabetes mellitus and proteinuria occur less commonly.14
Treatment of the acne related to PAPA syndrome can be difficult as it is poorly treatment responsive. The mainstays of treatment for the acne of PAPA syndrome are oral tetracycline and isotretinoin. Tumor necrosis factor a blockers and anakinra are used to treat the arthritis and pyoderma gangrenosum. The acne itself can be recurrent and need repeated courses of treatment.
FOLLICULAR OCCLUSION TETRAD
The follicular occlusion tetrad of cystic acne, hidradenitis suppurativa, dissecting cellulitis and pilonidal cysts can also manifest with acne vulgaris. Cystic acne itself can be treated and cured with isotretinoin and this should deal with co-existing acne vulgaris. Hidradenitis and dissecting cellulitis do not respond consistently to isotretinoin. Tumor necrosis factor a blockers are useful medical treatments for moderate and severe cases of hidradentitis. Radiation and radial surgery have been used for dissecting cellulitis but have associated morbidity. Pilonidal cysts can be excised or drained, but medical therapies are not that useful.
CONCLUSION
Acne is among the most common conditions that dermatologists treat. As this article has highlighted, it often occurs simultaneously with diseases such as seborrheic dermatitis and rosacea, and its treatment must account for this to optimize patient satisfaction and compliance.
A variety of conditions overlap with acne and affect the course and effectiveness of treatment. From a treatment standpoint, these conditions most importantly include seborrheic dermatitis and rosacea. From a diagnosis standpoint, important overlaps to recognize include the follicular occlusion tetrad (two or more of the following: cystic acne, hidradenitis suppurativa, dissecting cellulitis, pilonidal cysts), SAPHO syndrome and PAPA syndrome.
This article will review these acne overlaps and suggest therapeutic approaches.
SEBORRHEIC DERMATITIS
Seborrheic dermatitis is a condition that commonly overlaps with acne. Physically this can manifest as a combination of comedones and pustules on the face, chest and back, along with scaling erythematous plaques on the scalp, nasal alae and eyebrows. Hypopigmenation of the eyebrow and nasal alae skin can also be present.
The presence of co-incident sebborheic dermatitis has been suggested as an indicator for poor clinical response in acne patients treated with antibiotics and suggests early use of isotretinoin.1 Specifically, patients with a sebum excretion rate of 2.5 µgrams/cm2/minute experienced, on average, a 17% improvement with their acne in response to 6 months of treatment with topical 5% benzoyl peroxide and an oral agent from among one of the following: oral erythromycin (1 gram/daily), minocycline (100 milligram/daily), oxytetracycline (1 gram/daily) or cotrimoxazole (400 milligram/daily).
Topical Treatments For Seborrheic Dermatitis and Acne
A variety of topical treatments can be used to treat acne and seborrheic dermatitis simultaneously, including ketoconazole and sulfur.
Ketoconazole has antiandrogenic properties and might have utility against acne — which has an androgenic basis — not just seborrheic dermatitis.2
Sulfur has a keratolytic effect due to formation of hydrogen sulfide through a reaction that depends upon direct interaction between sulfur particles and keratinocytes and mild antifungal and antibacterial activity of unknown mechanism.3 Sulfur 5% and sodium sulfacetamide 10% alone or in combination are useful treatments for acne and seborrheic dermatitis.
Azelaic acid is an antikeratinizing agent, displaying antiproliferative cytostatic effects on keratinocytes and modulating the early and terminal phases of epidermal differentiation. It might be useful for sebborheic dermatitis, but there are no reports of azelaic acid use for seborrheic dermatitis.4
Antidandruff shampoos (e.g., tar, zinc, selenium) are helpful agents for this overlap, as they can allow for a treatment of the face and scalp with a wash and concentration of acne treatment of the face with leave-on products.
Oral Treatments For Seborrheic Dermatitis and Acne
Oral treatments can be useful for acne/seborrheic dermatitis overlap.
Isotretinoin is a useful, albeit not curative, treatment for seborrheic dermatitis. Very low-dose isotretinoin is effective in controlling seborrhea.5 Systemic isotretinoin reduces sebocyte lipid synthesis by 75% after 4 weeks, with daily doses as low as 0.1 mg/kg.
Oral ketoconazole has both antiandrogenic properties and is effective against pityrosporum folliculitis.7 This is the case in particular with women with hirsutism, acne and seborrheic dermatitis.8
Hormonal Treatments For Seborrheic Dermatitis and Acne
Hormonal treatments could have a role in treating the overlap of acne and seborrheic dermatitis. Estrogens decrease sebum production by suppressing gonadotrophin release and androgen production and increasing sex hormone-binding globulin production. There are a few studies that suggest that birth control pills with estrogens might be useful treatments for acne and seborrheic dermatitis.9 This is particularly true for the agents chlormadinone acetate, cyproterone acetate and dienogest (not available in the United States), potent, orally active progestogens, which have antiandrogenic, instead of partial androgenic, activity.10
Incompatible Acne/Seborrheic Dermatitis Treatments
Some treatments for acne and seborrheic dermatitis clash and should not be used when these conditions coincide.
Most importantly, topical steroids, which are helpful in the treatment of seborrheic dermatitis — likely by suppressing the inappropriate immune response to Malassezia furfur — but can cause acne. Thus, when these conditions overlap, topical steroids should not be used.
On a different note, antifungal agents that do not have anti androgenic effects — such as Allylamines — should not be used to treat acne/seborrheic dermatitis overlap.
ROSACEA
Classically it is stated that comedones do not manifest with rosacea. Sometimes, however, acne and rosacea do overlap. Rosacea is above all a disease of sensitive skin. This is important to be mindful of when treating acne/rosacea overlap.
Incompatible Acne and Rosacea Treatments
Acne Treatments. Some acne treatments are not appropriate for rosacea patients. Drying or irritating treatments for acne, such as benzoyl peroxide and salicyclic acid, are not well tolerated by patients with rosacea. Similarly, while trichloroacetic acid or beta peels can be used as adjuvants for acne treatment, they are extremely irritating in rosacea patients. Trimethoprim-sulfamethoxazole is a well-reported treatment for acne, but it is not used to treat rosacea, and hence should not be used for acne/rosacea overlap.
Rosacea Treatments. Likewise, there are treatments for rosacea that are not useful for acne and should be avoided in patients with acne/rosacea overlap. These include topical and oral metronidazole, topical tacrolimus or pimecrolimus and topical permethrin.
Appropriate Rosacea/Acne Combination Treatments
Although topical tretinoin is usually poorly tolerated by rosacea patients, adapalene can be tolerated by some rosacea patients and is useful in overlap patients. In fact, a comparison of topical adapalene gel (0.1%) and topical metronidazole gel (0.75%) found a similar effect against the inflammatory lesions of rosacea, but a superior effect in the metronidazole against erythema.11
Many treatments can be used in both acne and rosacea. In particular, oral tetracycline, azithromycin, doxycycline and minocycline are useful treatments for acne/rosacea overlap. Minocycline effectively decreases eyelid bacterial flora in patients with acne rosacea or blepharitis. One of the mechanisms of newer generation tetracycline analogues may be a decrease or elimination of bacterial flora from the eyelids.12 Isotretinoin can be curative for acne, and while only remittive for rosacea, is a very efficacious treatment while used. Similarly, sulfur 5% and sodium sulfacetamide 10%, alone or in combination, are useful treatments for acne and seborrheic dermatitis. Azelaic acid and topical clindamycin are useful for acne/rosacea overlap.
SAPHO
SAPHO syndrome is an acronym for a syndrome consisting in whole or part of synovitis, acne, pustulosis, hyperostosis, and osteitis. SAPHO is thought be an autoimmune disease in the family of psoriasis, rheumatoid arthritis and inflammatory bowel disease.
Joint manifestations of SAPHO mainly involve aseptic osteitis, osteomyelitis or arthritis of anterior chest, sacroiliac joints and long bones. Skin manifestations besides acne include palmoplantar pustulosis, non-palmoplantar pustulosis, psoriasis vulgaris, Sweet syndrome, hidradenitis suppurativa, dissecting cellulitis or pyoderma gangrenosum.
As the acne of SAPHO can be severe, the treatment of choice is isotretinoin; but as the stimulus underlying SAPHO is constant, repeated courses of therapy might be necessary.
PAPA SYNDROME
PAPA syndrome is an acronym for pyogenic arthritis, pyoderma gangrenosum and acne. PAPA syndrome is caused by mutations in proline serine threonine phosphatase-interacting protein [PSTPIP1, or CD2-binding protein 1 (CD2BP1)], a tyrosine-phosphorylated cytoskeletal organization protein interacting with pyrin. In this, it shares a common etiology with Familial Mediterranean Fever.13 Pyrin is a familial Mediterranean fever protein, cytoskeletal associated with myeloid/monocytic cells and modulates IL-1b, NF-kB activation and apoptosis.
An autosomal dominant disease with variable penetrance, PAPA syndrome can manifest in the following ways: (1) early pauciarticular, nonaxial, destructive, steroid-responsive arthritis; (2) pyoderma gangrenosum; (3) severe cystic acne in adolescence and beyond; and (4) acne vulgaris. In PAPA syndrome abscesses at the site of parenteral injections and cytopenia associated with sulfonamide medications, insulin-dependent, adult-onset diabetes mellitus and proteinuria occur less commonly.14
Treatment of the acne related to PAPA syndrome can be difficult as it is poorly treatment responsive. The mainstays of treatment for the acne of PAPA syndrome are oral tetracycline and isotretinoin. Tumor necrosis factor a blockers and anakinra are used to treat the arthritis and pyoderma gangrenosum. The acne itself can be recurrent and need repeated courses of treatment.
FOLLICULAR OCCLUSION TETRAD
The follicular occlusion tetrad of cystic acne, hidradenitis suppurativa, dissecting cellulitis and pilonidal cysts can also manifest with acne vulgaris. Cystic acne itself can be treated and cured with isotretinoin and this should deal with co-existing acne vulgaris. Hidradenitis and dissecting cellulitis do not respond consistently to isotretinoin. Tumor necrosis factor a blockers are useful medical treatments for moderate and severe cases of hidradentitis. Radiation and radial surgery have been used for dissecting cellulitis but have associated morbidity. Pilonidal cysts can be excised or drained, but medical therapies are not that useful.
CONCLUSION
Acne is among the most common conditions that dermatologists treat. As this article has highlighted, it often occurs simultaneously with diseases such as seborrheic dermatitis and rosacea, and its treatment must account for this to optimize patient satisfaction and compliance.
A variety of conditions overlap with acne and affect the course and effectiveness of treatment. From a treatment standpoint, these conditions most importantly include seborrheic dermatitis and rosacea. From a diagnosis standpoint, important overlaps to recognize include the follicular occlusion tetrad (two or more of the following: cystic acne, hidradenitis suppurativa, dissecting cellulitis, pilonidal cysts), SAPHO syndrome and PAPA syndrome.
This article will review these acne overlaps and suggest therapeutic approaches.
SEBORRHEIC DERMATITIS
Seborrheic dermatitis is a condition that commonly overlaps with acne. Physically this can manifest as a combination of comedones and pustules on the face, chest and back, along with scaling erythematous plaques on the scalp, nasal alae and eyebrows. Hypopigmenation of the eyebrow and nasal alae skin can also be present.
The presence of co-incident sebborheic dermatitis has been suggested as an indicator for poor clinical response in acne patients treated with antibiotics and suggests early use of isotretinoin.1 Specifically, patients with a sebum excretion rate of 2.5 µgrams/cm2/minute experienced, on average, a 17% improvement with their acne in response to 6 months of treatment with topical 5% benzoyl peroxide and an oral agent from among one of the following: oral erythromycin (1 gram/daily), minocycline (100 milligram/daily), oxytetracycline (1 gram/daily) or cotrimoxazole (400 milligram/daily).
Topical Treatments For Seborrheic Dermatitis and Acne
A variety of topical treatments can be used to treat acne and seborrheic dermatitis simultaneously, including ketoconazole and sulfur.
Ketoconazole has antiandrogenic properties and might have utility against acne — which has an androgenic basis — not just seborrheic dermatitis.2
Sulfur has a keratolytic effect due to formation of hydrogen sulfide through a reaction that depends upon direct interaction between sulfur particles and keratinocytes and mild antifungal and antibacterial activity of unknown mechanism.3 Sulfur 5% and sodium sulfacetamide 10% alone or in combination are useful treatments for acne and seborrheic dermatitis.
Azelaic acid is an antikeratinizing agent, displaying antiproliferative cytostatic effects on keratinocytes and modulating the early and terminal phases of epidermal differentiation. It might be useful for sebborheic dermatitis, but there are no reports of azelaic acid use for seborrheic dermatitis.4
Antidandruff shampoos (e.g., tar, zinc, selenium) are helpful agents for this overlap, as they can allow for a treatment of the face and scalp with a wash and concentration of acne treatment of the face with leave-on products.
Oral Treatments For Seborrheic Dermatitis and Acne
Oral treatments can be useful for acne/seborrheic dermatitis overlap.
Isotretinoin is a useful, albeit not curative, treatment for seborrheic dermatitis. Very low-dose isotretinoin is effective in controlling seborrhea.5 Systemic isotretinoin reduces sebocyte lipid synthesis by 75% after 4 weeks, with daily doses as low as 0.1 mg/kg.
Oral ketoconazole has both antiandrogenic properties and is effective against pityrosporum folliculitis.7 This is the case in particular with women with hirsutism, acne and seborrheic dermatitis.8
Hormonal Treatments For Seborrheic Dermatitis and Acne
Hormonal treatments could have a role in treating the overlap of acne and seborrheic dermatitis. Estrogens decrease sebum production by suppressing gonadotrophin release and androgen production and increasing sex hormone-binding globulin production. There are a few studies that suggest that birth control pills with estrogens might be useful treatments for acne and seborrheic dermatitis.9 This is particularly true for the agents chlormadinone acetate, cyproterone acetate and dienogest (not available in the United States), potent, orally active progestogens, which have antiandrogenic, instead of partial androgenic, activity.10
Incompatible Acne/Seborrheic Dermatitis Treatments
Some treatments for acne and seborrheic dermatitis clash and should not be used when these conditions coincide.
Most importantly, topical steroids, which are helpful in the treatment of seborrheic dermatitis — likely by suppressing the inappropriate immune response to Malassezia furfur — but can cause acne. Thus, when these conditions overlap, topical steroids should not be used.
On a different note, antifungal agents that do not have anti androgenic effects — such as Allylamines — should not be used to treat acne/seborrheic dermatitis overlap.
ROSACEA
Classically it is stated that comedones do not manifest with rosacea. Sometimes, however, acne and rosacea do overlap. Rosacea is above all a disease of sensitive skin. This is important to be mindful of when treating acne/rosacea overlap.
Incompatible Acne and Rosacea Treatments
Acne Treatments. Some acne treatments are not appropriate for rosacea patients. Drying or irritating treatments for acne, such as benzoyl peroxide and salicyclic acid, are not well tolerated by patients with rosacea. Similarly, while trichloroacetic acid or beta peels can be used as adjuvants for acne treatment, they are extremely irritating in rosacea patients. Trimethoprim-sulfamethoxazole is a well-reported treatment for acne, but it is not used to treat rosacea, and hence should not be used for acne/rosacea overlap.
Rosacea Treatments. Likewise, there are treatments for rosacea that are not useful for acne and should be avoided in patients with acne/rosacea overlap. These include topical and oral metronidazole, topical tacrolimus or pimecrolimus and topical permethrin.
Appropriate Rosacea/Acne Combination Treatments
Although topical tretinoin is usually poorly tolerated by rosacea patients, adapalene can be tolerated by some rosacea patients and is useful in overlap patients. In fact, a comparison of topical adapalene gel (0.1%) and topical metronidazole gel (0.75%) found a similar effect against the inflammatory lesions of rosacea, but a superior effect in the metronidazole against erythema.11
Many treatments can be used in both acne and rosacea. In particular, oral tetracycline, azithromycin, doxycycline and minocycline are useful treatments for acne/rosacea overlap. Minocycline effectively decreases eyelid bacterial flora in patients with acne rosacea or blepharitis. One of the mechanisms of newer generation tetracycline analogues may be a decrease or elimination of bacterial flora from the eyelids.12 Isotretinoin can be curative for acne, and while only remittive for rosacea, is a very efficacious treatment while used. Similarly, sulfur 5% and sodium sulfacetamide 10%, alone or in combination, are useful treatments for acne and seborrheic dermatitis. Azelaic acid and topical clindamycin are useful for acne/rosacea overlap.
SAPHO
SAPHO syndrome is an acronym for a syndrome consisting in whole or part of synovitis, acne, pustulosis, hyperostosis, and osteitis. SAPHO is thought be an autoimmune disease in the family of psoriasis, rheumatoid arthritis and inflammatory bowel disease.
Joint manifestations of SAPHO mainly involve aseptic osteitis, osteomyelitis or arthritis of anterior chest, sacroiliac joints and long bones. Skin manifestations besides acne include palmoplantar pustulosis, non-palmoplantar pustulosis, psoriasis vulgaris, Sweet syndrome, hidradenitis suppurativa, dissecting cellulitis or pyoderma gangrenosum.
As the acne of SAPHO can be severe, the treatment of choice is isotretinoin; but as the stimulus underlying SAPHO is constant, repeated courses of therapy might be necessary.
PAPA SYNDROME
PAPA syndrome is an acronym for pyogenic arthritis, pyoderma gangrenosum and acne. PAPA syndrome is caused by mutations in proline serine threonine phosphatase-interacting protein [PSTPIP1, or CD2-binding protein 1 (CD2BP1)], a tyrosine-phosphorylated cytoskeletal organization protein interacting with pyrin. In this, it shares a common etiology with Familial Mediterranean Fever.13 Pyrin is a familial Mediterranean fever protein, cytoskeletal associated with myeloid/monocytic cells and modulates IL-1b, NF-kB activation and apoptosis.
An autosomal dominant disease with variable penetrance, PAPA syndrome can manifest in the following ways: (1) early pauciarticular, nonaxial, destructive, steroid-responsive arthritis; (2) pyoderma gangrenosum; (3) severe cystic acne in adolescence and beyond; and (4) acne vulgaris. In PAPA syndrome abscesses at the site of parenteral injections and cytopenia associated with sulfonamide medications, insulin-dependent, adult-onset diabetes mellitus and proteinuria occur less commonly.14
Treatment of the acne related to PAPA syndrome can be difficult as it is poorly treatment responsive. The mainstays of treatment for the acne of PAPA syndrome are oral tetracycline and isotretinoin. Tumor necrosis factor a blockers and anakinra are used to treat the arthritis and pyoderma gangrenosum. The acne itself can be recurrent and need repeated courses of treatment.
FOLLICULAR OCCLUSION TETRAD
The follicular occlusion tetrad of cystic acne, hidradenitis suppurativa, dissecting cellulitis and pilonidal cysts can also manifest with acne vulgaris. Cystic acne itself can be treated and cured with isotretinoin and this should deal with co-existing acne vulgaris. Hidradenitis and dissecting cellulitis do not respond consistently to isotretinoin. Tumor necrosis factor a blockers are useful medical treatments for moderate and severe cases of hidradentitis. Radiation and radial surgery have been used for dissecting cellulitis but have associated morbidity. Pilonidal cysts can be excised or drained, but medical therapies are not that useful.
CONCLUSION
Acne is among the most common conditions that dermatologists treat. As this article has highlighted, it often occurs simultaneously with diseases such as seborrheic dermatitis and rosacea, and its treatment must account for this to optimize patient satisfaction and compliance.
