DOWLING-DEGOS

What is your diagnosis?

DOWLING-DEGOS DISEASE

Reticulated hyperpigmentation of the flexures

Comedone-like lesions on the back and neck

Pitted facial scars

Autosomal dominant with variable penetrance

Loss-of-function mutations in the gene that encodes keratin 5

The onset is typically during the third to fourth decade of life.

This increase in pign1entation is slowly progressive and initially affects the axillae and inguinal folds

Later in life, the intergluteal and inframammary folds, neck, trunk, and
inner aspects of the arms and thighs may become involved.

Comedone-like lesions on the back or neck, pitted perioral or facial scars, and epidermoid cysts are additional features.

An association with hidradenitis suppurativa (acne inversa) has been described

 Pathology

Histologically,

 there is increased pigmentation of the basal layer an
finger-like rete ridges with thinning of the suprapapillary epithelium.
 This results in an 'antler-like' pattern arising from the under surface of the epidermis and hair follicles.

Dermal melanophages and a perivascular lymphohistiocytic infiltrate are also observed.

Differential diagnosis

Differential diagnosis(reticulated pigmentation):

Galli galli diease(acantholitic variant of DDD)

Habers syndrom(rosacea like facial eruption plus the clinical feature of DDD)

Reticulated acro pigmentation of kitamura(atrophic acral lentigo-like lasions,palmoplantar and dorsal phalangeal pitting)

Pathology

Examination of the hyper pigmented macules shows:
 
 epidermal atrophy and elongated rete ridges that contain an increased amount of melanin.

An increased number of DOPA-positive melanocytes are seen in the basal layer of the epidermis.

Treatment

topical hydroquinone, tretinoin, adapalene and corticosteroids have been used with varying success.

Successful treatment with the erbium:YAG laser was reported in one patient.