Case 30

Female 76. Diabetes and thyroid disease. Multiple

crusty nodules on trunk. The best diagnosis is:

A.Excoriation

B.Spider bite

C.Lymphomatoid papulosis

D.Dermatitis artefacta

E.Reactive perforating collagenosis

Case 30

Female 76. Diabetes and thyroid disease. Multiple

crusty nodules on trunk.

Case 30

Female 76. Diabetes and thyroid disease. Multiple

crusty nodules on trunk.

Elastic van Gieson

Elastic van Gieson

Case 30. Reactive perforating collagenosis (Acquired perforating dermatosis): clinical features

• Male 60%. adults 29-96 yrs (mean 57). Limbs>trunk>head and neck.

• Pink/red papules enlarge and ulcerate/umbilicate with necrotic centre.

• Most show Koebner phenomenon, new lesions may arise from mild trauma. Lesions of various stages, most are pruritic, some severely.

• Majority associated with chronic renal failure especially on dialysis and/or diabetes mellitus, although idiopathic cases (such as this case) occur.

• characteristic thick purple ulcer slough on H+E.

• adjacent hyperplasia and hyperkeratosis.

• may see collagen and elastic fibres on H+E, or with connective tissue stain such as Masson’s trichrome & EVG.

• variable amount of connective tissue shows elimination: may be little/ none.

• variably associated with follicles

in the same patient.

Severe excoriation and trauma can show the same features and should always be considered in the differential diagnosis of perforating disorders.

Differential diagnosis: Excoriation Acquired perforating dermatosis

Severe excoriation and trauma including dermatitis arefacta can show the same features

should always be considered in the differential diagnosis of perforating disorders.

Differential diagnosis: Trauma reactive perforating collagenosis

Differential diagnosis: Spider bite Reactive perforating collagenosis

Differential diagnosis: lymphomatoid papulosis Reactive perforating collagenosis

Case 30. reactive perforating collagenosis (Acquired perforating dermatosis) :

differential diagnosis

Perforating folliculitis: Female>male, extremities and buttocks, intrafollicular curled up hair, collagen and elastic fibres eliminated, relapses and remits.

Case 30. reactive perforating collagenosis (Acquired perforating dermatosis) : differential diagnosis

• Elastosis perforans serpiginosa: age 10-25, most commonly on neck ,often arcuate/serpiginous group of papules, perforate elastic tissue, associated with connective tissue diseases eg Marfan’s, and penicillamine therapy.

• Kyrle‘s disease: mean 30yrs, especially lower limbs, small papules with central keratin plug, central invagination of epidermis containing keratin plug, often little/no perforation of collagen/elastin, also associated with diabetes mellitus and renal failure.

• Reactive perforating collagenosis (inherited type): 20-30yrs, upper and lower limbs & face, mainly perforation of collagen.

Case 30. reactive perforating collagenosis (Acquired perforating dermatosis) :

• Original classification based on nature of

eliminated material and type of

epidermal disruption but controversial

• currently the majority believe that perforating

disorders are a mixture of conditions that include

some specific entities such as the inherited forms

• however, acquired perforating disorders often resemble each other and the classical entities show much overlap

• hence the term “acquired perforating dermatosis” to encompass them:

• be descriptive rather than “shoe-horn” into any category.

• Since most show the Koebner phenomenon they may represent an abnormal response to minor trauma, and perforation is part of the healing process.

Case 30. reactive perforating collagenosis (Acquired perforating dermatosis) :

Treatment:

allopurinol, steroids, UVB, retinoids all shown variable effectiveness.

References

• 1. Saray Y, Seçkin D, Bilezikçi B. Acquired perforating dermatosis: clinicopathological features in twenty-two cases. J Eur Acad Dermatol Venereol. 2006 Jul;20(6):679-88.

• 2. Karpouzis A, Giatromanolaki A, Sivridis E, Kouskoukis C. Acquired reactive perforating collagenosis: current status. J Dermatol. 2010 Jul;37(7):585-92.

Case 30

Female 76. Diabetes and thyroid disease. Multiple

crusty nodules on trunk. The best diagnosis is:

A.Excoriation

B.Spider bite

C.Lymphomatoid papulosis

D.Dermatitis artefacta

E.Reactive perforating collagenosis