PASH syndrome: A case report with bony destruction. McCarthy, S., Foley, C., Dvorakova, V., Quinlan, C., Murphy, M., & Maher, M. Journal of the American Academy of Dermatology, 74(5):AB65, 2016.
PASH syndrome: A case report with bony destruction [link]Paper  abstract   bibtex   
A 44-year-old man with severe refractory hidradenitis suppurativa (HS) was admitted with cachexia and fatigue. He had a longstanding history of HS, beginning in childhood, cutaneous ulceration and acne. Since the age of 24 he had recurrent abscesses in the axillae and anogenital skin as well as cutaneous ulceration on the penile shaft, abdomen, forearms and thighs consistent with pyoderma gangrenosum (PG). On examination there was rope like dermal thickening of skin on the buttocks and perineum with florid erythema and multiple fistula openings with purulent discharge consistent with Hurley stage III disease. Large atrophic telangiectatic scars were present on the abdomen and thighs suggestive of quiescent pyoderma gangrenosum. CT scan of thorax, abdomen and pelvis showed lytic destruction of the sacrum most likely reflecting chronic osteomyelitis from direct extension of the inflammatory process through subcutaneous tissue and bone into the presacral space. The patient was treated with meropenem 1 g/day for seven days with marked improvement in HS. Inflammatory markers returned to normal and infliximab (0.5 mg/kg) was commenced to maintain remission. Hidradenitis suppurativa is a chronic autoinflammatory disorder characterized by acneiform follicular occlusion and inflammation of the terminal follicular epithelium in apocrine gland bearing skin that leads to relapsing purulent infections, scarring and sinus formation. The clinical presentation of severe HS, acne, pilonidal sinus and PG may be consistent with the recently recognised PASH syndrome. Our patient has never had joint symptoms suggestive of arthritis and therefore clinically does not fit with previously described PAPA (pyogenic sterile arthritis, PG, acne) or PAPASH (PAPA plus HS) syndromes. Imaging plays an important role in assessing extent of disease, characterizing active lesions and recognizing complications. To our knowledge this is the first case report of bony destruction of the coccyx by direct extension of HS.
@article{mccarthy_pash_2016,
	title = {{PASH} syndrome: {A} case report with bony destruction},
	volume = {74},
	issn = {0190-9622},
	url = {http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L72275278},
	abstract = {A 44-year-old man with severe refractory hidradenitis suppurativa (HS) was admitted with cachexia and fatigue. He had a longstanding history of HS, beginning in childhood, cutaneous ulceration and acne. Since the age of 24 he had recurrent abscesses in the axillae and anogenital skin as well as cutaneous ulceration on the penile shaft, abdomen, forearms and thighs consistent with pyoderma gangrenosum (PG). On examination there was rope like dermal thickening of skin on the buttocks and perineum with florid erythema and multiple fistula openings with purulent discharge consistent with Hurley stage III disease. Large atrophic telangiectatic scars were present on the abdomen and thighs suggestive of quiescent pyoderma gangrenosum. CT scan of thorax, abdomen and pelvis showed lytic destruction of the sacrum most likely reflecting chronic osteomyelitis from direct extension of the inflammatory process through subcutaneous tissue and bone into the presacral space. The patient was treated with meropenem 1 g/day for seven days with marked improvement in HS. Inflammatory markers returned to normal and infliximab (0.5 mg/kg) was commenced to maintain remission. Hidradenitis suppurativa is a chronic autoinflammatory disorder characterized by acneiform follicular occlusion and inflammation of the terminal follicular epithelium in apocrine gland bearing skin that leads to relapsing purulent infections, scarring and sinus formation. The clinical presentation of severe HS, acne, pilonidal sinus and PG may be consistent with the recently recognised PASH syndrome. Our patient has never had joint symptoms suggestive of arthritis and therefore clinically does not fit with previously described PAPA (pyogenic sterile arthritis, PG, acne) or PAPASH (PAPA plus HS) syndromes. Imaging plays an important role in assessing extent of disease, characterizing active lesions and recognizing complications. To our knowledge this is the first case report of bony destruction of the coccyx by direct extension of HS.},
	number = {5},
	journal = {Journal of the American Academy of Dermatology},
	author = {McCarthy, S. and Foley, C. and Dvorakova, V. and Quinlan, C. and Murphy, M. and Maher, M.},
	year = {2016},
	keywords = {American, abdomen, abscess, acne, apocrine gland, arthritis, autoinflammatory disease, axilla, bone, buttock, cachexia, case report, childhood, chronic osteomyelitis, coccygeal bone, computer assisted tomography, dermatology, epithelium, erythema, examination, fatigue, fistula, forearm, human, imaging, infection, inflammation, infliximab, male, marker, meropenem, occlusion, patient, pelvis, perineum, pilonidal sinus, pyoderma gangrenosum, remission, sacrum, scar, scar formation, skin, subcutaneous tissue, suppurative hidradenitis, thigh, thorax, ulcer},
	pages = {AB65}
}
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