Reactive perforating collagenosis in Hodgkin's disease

Two patients with Hodgkin's disease developed a condition that has been termed "reactive perforating collagenosis." In both cases, pruritus was severe and seemed to be responsible for the vigorous rubbing and scratching that caused the skin lesions. In one patient, the lesions disappeared upon remission of Hodgkin's disease. Perforating collagenosis has been described in children as a hereditary disease, and has also been described in adults with diabetes and on hemodialysis. On the basis of the changes seen in our two patients with Hodgkin's disease, we suggest that perforating collagenosis is simply a consequence of intense scratching.     

Acquired reactive perforating collagenosis

Acquired perforating disorder has been recognized as an uncommon distinct dermatosis in which altered collagen is eliminated through the epidermis. Several disorders accompanied by itching and scratching were reported to be associated with reactive perforating collagenosis. A 67-year-old white woman diagnosed as acquired reactive perforating collagenosis with poorly controlled diabetes mellitus and congestive cardiac failure is presented.     

Acquired reactive perforating collagenosis: Current status

Acquired reactive perforating collagenosis is a unique perforating dermatosis, characterized clinically by umbilicated hyperkeratotic papules or nodules and histologically by a focal hyperkeratosis in direct contact with transepidermal perforating dermal collagen. Several inflammatory or malignant systemic diseases may coexist with acquired reactive perforating collagenosis. The possible biochemical or immunological mechanisms of the systemic diseases, potentially responsible for the development and appearance of acquired reactive perforating collagenosis, are still under investigation. Several topical treatments, ultraviolet B phototherapy and allopurinol p.o. administration may be effective.     

Acquired perforating calcific collagenosis after topical calcium chloride exposure

A 24‐year‐old healthy man presented with a 6‐week history of numerous umbilicated coalescing erythematous papules with some scale and crust on his anterior medial thighs. The eruption began 1 to 2 weeks after he spilled calcium chloride rock salts on his pants while salting the sidewalk during a snow storm. The salts dissolved and remained in contact with his skin for at least 4 hours until he was able to change clothes. A skin biopsy shows thick and thin collagen fibers with partial calcification in the papillary and upper reticular dermis associated with a sparse infiltrate of neutrophils, lymphocytes and mononuclear histiocytes. There are foci of transepidermal elimination of calcified fibers with adjacent epidermal hyperplasia and ortho‐ and parakeratosis. Von Kossa stain highlights calcification of the fibers, and trichrome stain confirms the fibers are collagen. A Verhoeff‐van Gieson stain shows no abnormality of elastic fibers. The patient was treated with topical betametasone diproprionate cream twice daily for 3 weeks, as well as a short course of oral levofloxacin and topical gentamicin cream. The lesions resolved over 3 weeks with residual scarring. We report a unique case of acquired perforating calcific collagenosis secondary to topical calcium chloride exposure. Patel RR, Zirvi M, Walters RF, Kamino H. Acquired perforating calcific collagenosis after topical calcium chloride exposure.     

Acquired reactive perforating collagenosis in a patient with lung fibrosis

Reactive perforating collagenosis (RPC) is a rare disorder characterized by the transepidermal elimination of altered collagen. The inherited form of RPC begins in early childhood, but acquired reactive perforating collagenosis (ARPC) begins in adult life. ARPC is associated with diabetes mellitus, renal disease, and malignancy. ARPC with lung fibrosis has not previously been reported in the literature, and the relationship between ARPC and lung fibrosis has not been studied. The etiological relationship between the two disorders appears to be uncertain. Although their association in this case could be due to chance, it may be due to the transforming growth factor beta abnormalities seen in both diseases. In this report, we describe a case of ARPC with lung fibrosis and propose an etiological association between the two diseases.     

Acquired reactive perforating collagenosis in a diabetic patient with pulmonary aspergillosis

Reactive perforating collagenosis (RPC) is a rare disorder characterized by transepidermal elimination of altered collagen. The acquired form of RPC manifests in adult life and is accompanied by general pruritus and scratching. Diabetes mellitus and end-stage renal disease are frequently associated with RPC. We describe a case of RPC developing after pulmonary aspergillosis in a 54-year-old man with long-standing insulin-dependent diabetes and discuss possible etiologic relations.     

Reactive perforating collagenosis

Five cases of reactive perforating collagenosis (RPC) are reported. Four of these cases were in a father and his three children. This is the first reported instance of RPC occurring in more than one generation. The clinical and histological features of the disease are described and compared with other disorders showing perforating skin lesions, such as Kyrle's disease, perforating folliculitis and elastosis perforans serpiginosa. Evidence is presented to show that RPC is a genetically determined disorder with an inherited predisposition to an unusual skin reaction to superficial trauma.     

Acquired reactive perforating collagenosis following diclofenac therapy in a patient with parapsoriasis

A case of acquired reactive perforating collagenosis in a 70-year old man with a long standing digitate parapsoriasis is reported. Multiple asymptomatic cutaneous hyperpigmented papules with a central adherent keratotic plug developed simultaneously with widespread purpuric eruption following a single i.m. diclofenac injection. Histologic examination of a keratotic lesion revealed an epidermal crater with centrally degenerated collagen and elastic fibre extrusion. This case is interesting for the lack of pruritus, the transepithelial elimination of both collagen and elastic fibres and the association with parapsoriasis. The etiologic role of diclofenac is discussed. The term acquired perforating dermatosis may be more suitable for this case.