HPI: The patient is otherwise well and has been treated with doxycycline for greater than five years for severe rosacea. Recently, it has not been effective. He presented for alternative therapy; and at the time of the visit he mentioned an a pruritic papular eruption of his thighs for two to three weeks. He has had recent onset hypertension and was started on HCTZ about a month ago.
O/E: Erythematous papules and small nodules on face. There are scattered three - four mm papules erythematous papules on the medial thighs. The remainder of the examination is unremarkable.
Clinical Photos:
Subtle Lesions on Thighs
Pathology: Necrotizing vasculitisof deep dermal artery. (Photomicrographs courtesy of Marjan Mirzabeiji, M.D., Boston University Department of Dermatology, Dermatopathology Section)
Lab: CBC normal, Chemistries normal, BUN/Cr normal, ANA 1:1280 Homogenous, ANCA panel negative
Diagnosis: Cutaneous Polyarteritis Nodosa (drug-induced) or microscopic polyangiitis. Doxycycline or HCTZ may be putative.
Discussion: This is an "interesting" case. A man walks in with rosacea and winds up with necrotizing vasculitis. He has some protein in his urine and a positive ANA. There's an old saying: It is often more important to treat the patient who has the disease than the disease the patient has. This may be a case in point.
Questions: What is your diagnosis and what more would you do?
Reference:
Rogalski C, Sticherling M. Panarteritis cutanea benigna--an entity limited to the skin or cutaneous presentation of a systemic necrotizing vasculitis? Report of seven cases and review of the literature. Int J Dermatol. 2007 Aug;46(8):817-21
Abstract: In 1931 Lindberg described a limited and benign subcutaneous form of panarteritis nodosa, which, in contrast to systemic panarteritis, only affects the skin. The terms panarteritis nodosa cutanea benigna, cutaneous polyarteritis nodosa, apoplexia cutanea Freund as well as livedo with nodules are used synonymously for this vasculitis which predominantly affects women in the fifth decade of life. Cutaneous lesions characteristically comprise painful subcutaneous nodules or vasculitis racemosa at the lower extremities. The cutaneous panarteritis may be regarded as its own entity or an isolated skin manifestation within systemic panarteritis nodosa. Full Abstract.
Great biopsy! With a relatively asymptomatic patient and only 2 medications, I'd stop the doxy and HCTZ for a month. If that works, fine. If the condition doesn't reverse itself, I might even try dapsone.
ReplyDeleteFrom Andrew Carlson, Department of Dermatopathology, Albany Medical Center: "Where was the biopsy taken- thigh or face?
ReplyDeleteBased on clinical and laboratory findings, I would say he has an indolent variant of systemic polyarteritis nodosa (idiopathic or secondary to drug or CTD). One wonders how his severe rosacea (chronic inflammation) was responsible for promoting vasculitis.
The possibility of giant cell arteritis should also be considered.
(The bx was taken from the thigh)